Kaplan Questions Flashcards
(108 cards)
Describe the rash associated with erythema infectiosum
aka Fifth disease is a viral infection by human parvovirus B19 that is mildly contagious and affects children 3-12 yrs. An erythematous malar rash appars –> erythematous maculopapular eruption on the extensor extremities the next day. When the rash fades a few days later, areas of central clearing leave behind a reticulated, lacy patterned rash thatwaxes and wanes for an avg of 9-11 days.
Wiskott aldrich is an X-linked disease characterized by a triad of eczema, thrombocytopenia and recurrent infections. Child has increased risk of?
Lymphoma inf they survive past 15 since they often have severe infections
Gaucher disease -AR lysosomal storage disease caused by deficiency of glucocerebrosidase (aka glucosylcermidase): what are the symptoms?
- asymptomatic painless HSM possibly with elevated transaminases
- thrombocytopenia
- anemia
- BONE abnormalities (pain crisis, avascular necrosis, osteoporosis, fractures and even abnormal modeling with widening of the distal femurs looks like ERLENMEYER FLASK!)
JIA -worry about which most feared complication?
do slit lamp to screen for iridocyclitis
Which congenital heart disease is assoc with?
- cyanosis
- systolic murmur
- a single S2
- CXR: normal heart size and increased pulmonary vascular markings (“SNOWMAN”)
- EKG: RVH, peaked p waves
- ABG: respiratory acidosis
Total anomalous pulmonary venous return when pulmonary veins drain into the right atrium –> right atrial enlargement (spiked p waves)
IF you suspect shaken baby syndrome, what should be done before reporting to CPS?
bleeding disorders should be ruled out so get coag studies
Acute osteo is inflammation of bone marrow caused by infection that is rapid and usu severe. Symptoms include fever, refusal to bear weight or ambulate with a limp and localized pain and tenderness. Most common causative agent in children?
Staph aureus
most commonly via hematogenous spread
When a premature infant develops obstructive hydrocephalus with symptoms like poor feeding, irritability, reduced activity, tense anterior fontanelle, distended scalp veins, sunset sign (eyeballs are deviated downward and upper lids are retracted), suspect!?
Germinal matrix hemorrhage aka intraventricular hemorrhage. The GM is a highly vascularized layer of neuroectodermal precursors lining the ventricular cavities. These vessels are very vulnerable to hypoxic insults.
Kawasaki Disease aka mucocutaneous LN syndrome -a systemic vasculitis of unknown etiology. List symptoms and treatment and what to worry about
Diagnostic criteria:
- fever >/- 5 days often to 104
- b/l nonexudative conjunctival injection
- changes of the oral mucosa, injected pharynx, or strawberry tongue
- polymorphic exanthem of the trunk
- changes in hands and feet including redness, swelling
Do screen for heart conditions like coronary artery aneurysms
Treat with IVIG and high-dose aspirin
Which acid-base condition is assoc with pyloric stenosis?
vomiting so metabolic alkalosis
Cystinuria -AR d/o affecting reabsoprtion of COAL (cystine, ornithine, arginine, lysine) –> urolithiasis
What will you see on UA? How to diagnose?
Hexagonal crystals on UA are characteristic of cystine crystals. And dx is confirmed with a 24-h urine for cystine
How to differentiate btw congenital hemophilia A with low factor VIII levels vs acquired hemophilila A 2/2 to anti-factor VIII abs?
plasma mixing study
- add plasma with factor VIII into pt’s serum
- if PTT improves, then it’s congenital, but if it doesn’t, then it’s acquired bc the added VIII will still be attacked by the antibodies
vWF deficiency -how to diagnose? This is defect in both plt aggregation as vWF acts like a glue to bind plts to endothelium and clotting factor activity (as vWF is the cofactor of factor VIII). What will you see in labs?
Ristocetin cofactor activity -tests the ability of vWF to agglutinate plts by binding to its primary receptor, GP1b
Bleeding time is prolonged b/c of decreased plt functions
Increased PTT b/c vWF serves as cofactor for VIII
Jones Criteria for Rheumatic Fever: Dx requires 2 major or 1 major + 2 minor symptoms
Major symptoms: Carditis, Erythema marginatum and subcutaneous nodules, Sydenham’s chorea, arthritis
Minor symptoms: Fever, polyarthralgia, reversible prolongation of the PR interval, Increased ESR, recent beta-hemolytic strep infection, h/o of rheumatic fever
T1DM has increased risk of which GI disease?
celiac disease
-should get IgA and tissue transglutaminase antibody
Ebstein anomaly -what with CXR show
ebstein anomaly is when there’s a hypoplastic RV and enlarged RA b/c of tricuspid atresia. CXR will show cardiomegaly and and decreased pulmonary vasculature
Chronic granulomatous disease -def of NADPH oxidase that causes inadequate production of H2O2, superoxide and other O2 species in neutrophils. What’s the test?
nitroblue tetrazolium dye reduction test will confirm defective neutrophils inability to produce these o2 species necessary in killing of bacteria (staph, burkholderia, pseudomona, serratia, nocardia, aspergillus)
What does monoclonal anti-CD11 help diagnose?
LAD deficiency in which the CD11 antigen on surface of WBCs is missing. This can lead to delayed cord healing
What’s the most common bacterial infection causing CNS infections in those who have neurosurg procdures?
staph epidermidis produces biofilm that allows them to adhere to surfaces of medical prostheses.
will need vancomycin
Roseola infantum due to HHV6 and 7
maculopapular rash that spreads from trunk to extremities and is assoc with resolution of high fevers
If you discover a mass of SCM muscle after birth -it is most commonly?
congenital torticollis aka SCM tumor of infancy that will regress in 4-8 months
NF-1: symptoms
cafe-au-lait macules neurofibromas freckling in axillary region optic glioma iris hamartomas osseous lesion/BONY dysplasia
Tuberous sclerosis: symptoms
cardiac rhabdomyoma ash-leaf macules (hypopigmented macules) facial angiofibromas (adenoma sebaceum) Shagreen patches (CT nevi) Seizures (most common presenting symptom)
Sturge Weber: symptoms
cerebral calcificatiosn (train tracks) facial nevus port wine stain glaucoma in ipsilateral eye seizures c/l to side of nevus made with skull x-ray or CT showing calcifications